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Renal Disease in Cryoglobulinemia
Thomas Menter1, Helmut Hopfer1
1Pathology, Institute for Medical Genetics and Pathology, University Hospital Basel, University of Basel, Basel, Switzerland.
Glomerular Diseases
|February 8, 2023
Summary
Diagnosing renal disease in cryoglobulinemia is challenging due to its rarity and overlapping morphologies. This study highlights key diagnostic features on kidney biopsies, emphasizing electron microscopy findings for accurate cryoglobulinemia diagnosis.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Renal disease in cryoglobulinemia presents diagnostic challenges due to rarity, difficult serological testing, and overlapping morphologies with other kidney diseases.
- A definitive diagnosis can be elusive even after advanced microscopic studies, necessitating a high index of suspicion.
Purpose of the Study:
- To discuss and illustrate diverse morphological patterns of renal injury in cryoglobulinemia using kidney biopsy data.
- To outline pathophysiologic, clinical, and electron microscopic features of cryoglobulinemia-induced renal disease.
- To present a practical, morphology-based diagnostic approach for renal biopsies, considering differential diagnoses.
Main Methods:
- Analysis of kidney biopsies from routine diagnostic and referral practice.
- Illustration of morphological patterns of renal injury.
- Focus on electron microscopy for detailed morphologic changes.
- Consideration of differential diagnoses and disease mimickers.
Main Results:
- Cryoglobulinemia-associated glomerulonephritis (GN) shows membranoproliferative GN with abundant monocytes and (pseudo)thrombi.
- Electron microscopy reveals prominent monocyte/macrophage infiltration and mesangial/subendothelial deposits.
- Distinctive features include curved microtubular/cylindrical and annular substructures within deposits.
Conclusions:
- Recognizing cryoglobulinemia-associated GN is crucial for clinical practice.
- Pathologists and clinicians must maintain a high suspicion for cryoglobulinemia in renal biopsies, as it's often not tested beforehand.
- A morphology-based approach, particularly utilizing electron microscopy, aids in diagnosing this challenging condition.
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